Type I primary hyperoxaluria is a rare autosomal recessive disorder of gloxylate matabolism that ultimately leads to chronic renal insufficiency.1 While numerous forms of therapy have been tried, none has been shown to halt the inexorable progression of the renal lesion. We report a patient with renal failure secondary to primary hyperoxaluria. The patient received a renal transplant, and we review the literature on transplantation in this metabolic disorder.
—A 30-year-old white man was transferred to the Peter Bent Brigham Hospital on March 30,1971, to be evaluated for long-term hemodialysis and kidney transplantation. When two years old, he first passed a renal stone. Renal colic recurred when the patient was 12 years old and in the ensuing years he passed numerous stones. When he was 18 years old, the patient had a blood urea nitrogen (BUN) concentration of 50 mg/100 ml and bilateral nephrolithiasis. In